cellular
• lysis of erythrocytes
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hematopoietic system
• twice as large as wild-type
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• marked erythroid hyperplasia
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• reticulocytosis with an increase in red cell distribution width (RDW), reflecting the size variation
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• Heinz bodies, reflecting precipitated hemoglobin are present
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• young red blood cells, with erythrocyte shape irregularities and occasional inclusions
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homeostasis/metabolism
• reduced catalase activity in erythrocytes
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• in a methylcellulose colony assay the formation of burst-forming unit erythroid (BFU-e)-type colonies from
mutant bone marrow progenitors is inhibited selectively by buthionine sulfoximine (BSO), a pro-oxidant that depletes cells of glutathione
• exhibit a more rapid and profound decreases in hematocrit levels and subsequent delayed recovery in response to
phenylhydrazine,(PHZ), an oxidant that induces denaturation of hemoglobin, resulting in rapid erythrocyte destruction
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immune system
• twice as large as wild-type
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• marked erythroid hyperplasia
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growth/size/body
• twice as large as wild-type
|
• marked erythroid hyperplasia
|